Adrenocortical carcinoma (ACC) is an aggressive malignancy that forms in the adrenal cortex. ACC generally occurs in adults with a median age of diagnosis at 44 years. ACC is curable when detected early, but because most ACC tumors are still capable of functioning, many patients do not present with endocrine symptoms. As a result, between 40% and 70% of patients have metastatic disease at the time of diagnosis and cannot be cured by surgery. Further, mitotane, the only drug compound approved for treatment of ACC, has severe, dose limiting side effects and only shows a response in approximately 22% of patients. The combination of late diagnosis and ineffective treatments for those with disseminated disease results in an overall 5-year survival rate of 10-20%.
Factors that predict the outcome of a patient's disease are a form of personalized medicine that can inform health care providers about individual patient treatment. Patients likely to have a poorer outcome may have the option of undertaking an aggressive treatment regimen including chemotherapy or experimental treatments. Efforts have been made to seek prognostic factors such as hypo- and hyperdiploidy and expression profiling. While these may be used to manage treatment of ACC patients, they are not a single gene prognostic test. A single gene prognostic test could be more readily adopted by health care providers.